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A 27-year-old Japanese woman with a history of depression and an eating disorder presented to our emergency department with a chief complaint of generalized weakness. Electrocardiography showed prominent QT prolongation with multiple ventricular contractions. Chest X-ray plain computed tomography revealed pulmonary edema. Echocardiography showed decreased left ventricular systolic function. Suspecting acute myocarditis, we performed a myocardial biopsy from the right ventricular septum. The biopsy histology revealed extensive myocardial fibrosis and a very mild inflammatory cell infiltrate. In an additional detailed medical interview, the patient admitted that she had consumed three bottles of a first-aid liquid containing naphazoline approximately ~12 h before her presentation, in a suicide attempt. Her QTc and left ventricular ejection fraction improved during hospitalization. Acute drug intoxication can cause QT prolongation and ventricular arrhythmias, cardiomyopathy, and pulmonary edema. When acute QT prolongation, myocardial damage, and pulmonary edema are seen (suggesting acute myocarditis), naphazoline intoxication should be investigated in the differential diagnosis. [ABSTRACT FROM AUTHOR]

Cardiac cysticercosis is rare in clinical practice and is usually accidentally identified during cardiac surgery or autopsies. Although mostly asymptomatic, cardiac cysticercosis could present with severe clinical conditions such as myocarditis, acute myocardial infarction, and arrhythmia. We present a 51-year-old female patient who accidentally discovered a solitary mass in the myocardium. The cardiac magnetic resonance imaging revealed a nonenhanced cyst in the interventricular septum protruding into the right ventricular chamber. Because of cardiac tamponade presenting during a right ventricular endomyocardial biopsy, an emergency open-heart surgery was performed to suture the ventricular wall perforation and remove the tumor. The histopathologic report demonstrated typical cysticercosis. Cardiac cysticercosis is an uncommon lesion and may present with atypical clinical and laboratory features. Therefore, this diagnosis should be considered single or multiple cardiac cystic lesions.

Aim. To assess the incidence of myocarditis in patients with primary myocardial hypertrophy and to study its contribution to the disease course.Material and methods. The study included 100 patients with primary left ventricular myocardial hypertrophy, 52 men and 48 women (mean age, 51,5±15,7 years; followup period 10,4 [2,1; 36,1] months). All patients underwent electrocardiography, 24-hour electrocardiographic monitoring, echocardiography, as well as DNA analysis (n=96), myocardium pathological study (n=29), cardiac magnetic resonance imaging (n=31), cardiac multislice computed tomography (n=26), assessment of anti-cardiac antibodies (n=43), free light chain level in serum and urine by immunofixation method (n=10); 99mTc-pyrophosphate myocardial scintigraphy (n=5); biopsy of the rectal mucosa and/or subcutaneous fat for amyloid (n=9).Results. In 68%, true (sarcomeric) hypertrophic cardiomyopathy (HCM) was diagnosed, in 16% — amyloidosis with cardiac involvement, in 10% — storage diseases, in 3% — neuromuscular diseases, in 2% — myocardial hypertrophy was combined with severe restriction (mixed phenotype), and in 1% — LEOPARD syndrome. Concomitant myocarditis was diagnosed in 30% of patients. In HCM, myocarditis was detected in 31% of cases. These patients had a significantly higher heart failure class (heart failure class 3 [2; 3] vs 2 [1; 3], p=0,026) and mortality (33,3% vs 6,4%, p=0,01). In amyloidosis, the incidence of myocarditis was 31,3%. In these patients, ventricular tachycardia was observed significantly more often: 80,0% vs 18,2% (p=0,036). The prevalence of concomitant myocarditis in the subgroup of storage diseases was 30%: 2 patients with Fabry disease and 1 patient with Danon disease. Of the three patients with neuromuscular diseases, myocarditis was diagnosed in 1. In the subgroups with the restrictive phenotype and LEOPARD syndrome, no cases of myocarditis were recorded. Treatment of myocarditis made it possible to stabilize the patients' condition.Conclusion. Concomitant myocarditis led to heart failure progression, worsening ventricular arrhythmias and, as a consequence, an increased risk of sudden cardiac death. It is necessary to actively diagnose and treat myocarditis in patients with primary myocardial hypertrophy.

Arrhythmias occur both in the acute coronavirus disease 2019 (COVID-19) and in the post-acute period, which may be associated with the long-term SARS-CoV-2 persistence. In a case report, patient with primary dilated cardiomyopathy, an implanted cardioverter-defibrillator, recurrent ventricular arrhythmias, and an electrical storm are presented. The patient was repeatedly hospitalized in a city hospital, where electrical cardioversion, antiarrhythmic therapy, radiofrequency ablation of recurrent ventricular tachycardia regions, including emergency ("lifesaving") combined catheter radiofrequency destruction of the electrical storm substrate, was performed. Subsequently, against the background of ongoing postoperative therapy, massive pulmonary embolism (PE) suddenly developed, which led to the death. Autopsy established that the cause of the patient’s death was massive pulmonary embolism; SARS-CoV-2 and enterovirus was detected in the myocardium. Based on an autopsy study, the patient was diagnosed with primary dilated cardiomyopathy with secondary active chronic lymphocytic myocarditis.

In a myocardial biopsy, the physician inserts the myocardial biopsy forceps into the heart chamber with fluoroscopic guidance and operates the forceps to collect the myocardium. The tip of the forceps has a gripper and a two-degree-of-freedom passive joint. The myocardial wall is difficult to see through a fluoroscopic image, and sampling may be missed due to the weak contact force and the large angle between the forceps and the myocardium. To solve these problems, we propose a contact force and bending angle detection mechanism for the forceps using optical fibers. The proposed mechanism has three built-in optical fibers and is connected to light-intensity-modulation-type optical sensors. Before contact, the light of the fibers leaks outside through the forceps shaft gap. When the forceps tip comes into contact with the myocardial wall, the external force reduces the shaft gap and the leak light amount. The physician can detect the contact force and bending state at the tip from the ratio of the three measured values. We evaluated the proposed mechanism in in vitro and in vivo experiments. We confirmed that the contact force under 1.4 N and bending angle over 30 deg can be detected. [ABSTRACT FROM AUTHOR]

An 83-year-old man with a 5-month history of asthma presented to the emergency department with chest oppression and dyspnea. Electrocardiography showed ST-segment depression. Transthoracic echocardiography showed no asynergy with an ejection fraction of 62%. Coronary angiography revealed no stenosis. On day 3, he developed worsening dyspnea, cough, and rapidly progressive acute decompensated heart failure with abdominal purpura and lower extremity petechiae.Myocardial and skin biopsies revealed eosinophilic infiltration. He was diagnosed with acute eosinophilic myocarditis and heart failure due to eosinophilic granulomatosis with polyangiitis. Methylprednisolone pulse therapy dramatically improved his symptoms and congestion. Dual single-photon emission computed tomography after 1 year demonstrated lesion improvement.

Grigorios Korosoglou,1,2 Sorin Giusca,1,2 Florian André,3,4 Fabian aus dem Siepen,3,4 Peter Nunninger,5 Arnt V Kristen,3,6 Norbert Frey3,4 1GRN Hospital Weinheim, Department of Cardiology, Vascular Medicine and Pneumology, Weinheim, Germany; 2Cardiac Imaging Center Weinheim, Hector Foundation, Weinheim, Germany; 3Department of Cardiology, Pneumology and Angiology, University Hospital Heidelberg, Heidelberg, Germany; 4German Centre for Cardiovascular Research (DZHK), Partner Site Heidelberg, Heidelberg, Germany; 5Radiology and Nuclear Medicine Weinheim, Weinheim, Germany; 6Cardiovascular Center Darmstadt, Darmstadt, GermanyCorrespondence: Grigorios KorosoglouGRN Hospital Weinheim, Department of Cardiology & Vascular Medicine, Roentgenstrasse 1, Weinheim, D-69469, GermanyTel +49 6201 89 2142Fax +49 6201 89 2507Email gkorosoglou@hotmail.comAbstract: Among non-ischemic cardiomyopathies, cardiac amyloidosis is one of the most common, being caused by extracellular depositions of amyloid fibrils in the myocardium. Two main forms of cardiac amyloidosis are known so far, including 1) light-chain (AL) amyloidosis caused by monoclonal production of light-chains, and 2) transthyretin (ATTR) amyloidosis, caused by dissociation of the transthyretin tetramer into monomers. Both AL and ATTR amyloidosis are progressive diseases with median survival from diagnosis of less than 6 months and 3 to 5 years, respectively, if untreated. In this regard, death occurs in most patients due to cardiac causes, mainly congestive heart failure, which can be prevented due to the presence of effective, life-saving treatment regimens. Therefore, early diagnosis of cardiac amyloidosis is crucial more than ever. However, diagnosis of cardiac amyloidosis may be challenging due to variable clinical manifestations and the perceived rarity of the disease. In this regard, clinical and laboratory reg flags are available, which may help clinicians to raise suspicion of cardiac amyloidosis. In addition, advances in cardiovascular imaging have already revealed a higher prevalence of cardiac amyloidosis in specific populations, so that the diagnosis especially of ATTR amyloidosis has experienced a > 30-fold increase during the past ten years. The goal of our review article is to summarize these findings and provide a practical approach for clinicians on how to use cardiovascular imaging techniques, such as echocardiography, cardiac magnetic resonance, bone scintigraphy and, if required, organ biopsy within predefined diagnostic algorithms for the diagnostic work-up of patients with suspected cardiac amyloidosis. In addition, two clinical cases and practical tips are provided in this context.Keywords: cardiac amyloidosis, ATTR amyloidosis, AL amyloidosis, echocardiography, cardiac magnetic resonance, bone scintigraphy, myocardial biopsy, specific therapy

Objectives: The purpose of this study was to assess osimertinib-associated cardiac adverse events (AEs) in a real-world setting, using a retrospective single-center cohort study in Japan. Background: Cases of osimertinib-associated cardiac AEs have been reported but remain poorly understood. Methods: A total of 123 cases of advanced non–small cell lung cancer (NSCLC) with confirmed EGFR mutations who received osimertinib monotherapy from 2014 to 2019 at the Osaka International Cancer Institute (Osaka, Japan) were evaluated. Cardiac AEs were defined according to Common Terminology Criteria for Adverse Events (CTCAE) version 5.0. Changes in left ventricular ejection fraction (LVEF) and rates of cancer therapeutics-related cardiac dysfunction (CTRCD), defined as a ≥10 % absolute decline in LVEF from baseline to a value of

Objectives: Fulminant myocarditis (FM) is a rapidly progressive and frequently fatal form of myocarditis that has been difficult to classify. This study aims to compare the clinical characteristics, treatments and outcomes in patients with fulminant giant cell myocarditis (FGCM) and fulminant lymphocytic myocarditis (FLM).Methods and Results: In our retrospective study, nine patients with FGCM (mean age 47.9 ± 7.5 years, six female) and 7 FLM (mean age 42.1 ± 12.3 years, four female) patients confirmed by histology in the last 11 years were included. Most patients with FGCM and FLM were NYHA functional class IV (56 vs. 100%, p = 0.132). Patients with FGCM had significantly lower levels of high-sensitivity C-reactive protein [hs-CRP, 4.4 (2.0–10.2) mg/L vs. 13.6 (12.6–14.6) mg/L, P = 0.004, data shown as the median with IQR], creatine kinase-myoglobin [CK-MB, 1.4 (1.0–3.2) ng/ml vs. 14.6 (3.0–64.9) ng/ml, P = 0.025, median with IQR], and alanine aminotransferase [ALT, 38.0 (25.0–61.5) IU/L vs. 997.0 (50.0–3,080.0) IU/L, P = 0.030, median with IQR] and greater right ventricular end-diastolic diameter (RVEDD) [2.9 ± 0.3 cm vs. 2.4 ± 0.6 cm, P = 0.034, mean ± SD] than those with FLM. No differences were observed in the use of intra-aortic balloon pump (44 vs. 43%, p = 1.000) and extracorporeal membrane oxygenation (11 vs. 43%, p = 0.262) between the two groups. The long-term survival rate was significantly lower in FGCM group compared with FLM group (0 vs. 71.4%, p = 0.022). A multivariate cox regression analysis showed the level of hs-CRP (hazard ratio = 0.871, 95% confidence interval: 0.761–0.996, P = 0.043) was an independent prognostic factor for FM patients. Furthermore, the level of hs-CRP had a good ability to discriminate between patients with FGCM and FLM (AUC = 0.94, 95% confidence interval: 0.4213–0.9964).Conclusions: The inflammatory response and myocardial damage in the patients with FGCM were milder than those with FLM. Patients with FGCM had distinctly poorer prognoses compared with those with FLM. Our results suggest that hs-CRP could be a promising prognostic biomarker and a hs-CRP level of 11.71 mg/L is an appropriate cutoff point for the differentiating diagnosis between patients with FGCM and FLM.

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe drug reaction characterized by skin rash, organ involvement, lymph node swelling, eosinophilia, and atypical lymphocytosis, with myocarditis being a rare but potentially fatal complication. It has been reported that in patients with cardiac involvement due to DRESS, older age and shorter periods between offending drug exposure and symptom onset are associated with mortality. We report a case of fatal DRESS-associated myocarditis in a young woman, occurring one month after drug exposure, despite intensive immunosuppressive therapy. This case report highlights the risk of mortality from DRESS-associated myocarditis even in patients lacking known risk factors., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Watanabe et al.)

Competing Interests: The authors declare no conflicting interests.

We aim to evaluate the value of Cardiac magnetic resonance (CMR) feature tracking (CMR-FT) in addition to Task Force Criteria(TFC) in patients with (arrhythmogenic cardiomyopathy) AC biopsy-proved. Thirty-five patients with AC histologically proven who performed CMR with late gadolinium enhancement (LGE) acquisition were enrolled. The study population was divided in Group1 (negative CMR TFC and LV ejection fraction≥55%) and Group2 (positive CMR TFC and/or LVEF<55%) and compared to an age and gender-matched control group. CMR datasets of all patients were analyzed to calculate LV indexed end-diastolic (LVEDi) and end-systolic (LVESi) volumes and RV indexed end-diastolic (RVEDi) and end-systolic (RVESi) volumes, both LV ejection fraction (LVEF) and RV ejection fraction (RVEF). Moreover, LV and RV global longitudinal (GLS), circumferential (GCS) and radial (GRS) strain were measured. The AC patients showed both higher LVEDi (p:0.002) and RVEDi (p:0.017) and lower LVEF (p: 0.016) as compared to control patients. Moreover, AC patients showed impaired LV-GLS (p < 0.001), LV-GRS (p < 0.001), LV-GCS (p < 0.001) and RV-GRS (p:0.026) as compared to control subjects. Group1 patients showed a significant reduction of LV-GRS (p < 0.05) and LV-GCS p < 0.01) as compared to control subjects. At univariate analysis LV-GCS was the most discriminatory parameter between Group1 vs heathy subjects with an optimal cut-off of −15.8 (Sensitivity: 74%; Specificity: 10%). In patients with AC biopsy-proven, CMR-FT could improve the diagnostic yield in the subset of patients who results negative for imaging TFC criteria resulting as useful gatekeeper for indication of myocardial biopsy in case of equivocal clinical and imaging presentation. • Evaluation of strain could be helpful in management of patients with suspected arrhythmogenic cardiomyopathy. • Strain can be fundamental for identification of patients that need myocardial biopsy • Strain could identify patients with early involvement of arrhythmogenic cardiomyopathy. [ABSTRACT FROM AUTHOR]

Multisystem Inflammatory Syndrome (MIS) is a novel hyperinflammatory syndrome associated with SARS-CoV-2 infection. It predominantly affects children (MIS-C) a few weeks after a usually asymptomatic SARS-CoV-2 infection and is only rarely seen in adults above 21 years (MIS-A). Only scarce data on histological findings in both pediatric and adult patients has been published so far. An 18-year-old male patient was admitted to hospital in a febrile state, which progressed to severe cardiogenic shock and multi-organ failure requiring extracorporeal life support. Myocardial biopsy revealed small vessel-associated immune cell infiltrates. Diagnosis of MIS-C was made after ruling out all potential differential diagnosis. Use of immunosuppressive treatment with steroids, interleukin-1 blockade and high-dose intravenous immunoglobulins resulted in the patient's full recovery. Multisystem Inflammatory Syndrome (MIS) is a new differential diagnosis of cardiac dysfunction in pediatric and adult patients. The lack of myocardial necrosis differentiates the disease from other viral myocarditis and offers an explanation for the fast response to immunomodulatory therapy and the favorable prognosis. The preceding SARS-CoV-2 infection might only have been mildly symptomatic or even asymptomatic.

Aim. To study the nosological spectrum in the syndrome of primary left ventricle hypertrophy (PLVH) using morphological and genetic diagnostics and to compare the clinical course of true hypertrophic cardiomyopathy (HCM) and its phenocopy.Material and methods. Fifty five adult patients (29 men, 48.2±17.0 years) with PLVH (12 mm and more) were included. The exclusion criteria were athletic heart, hypertensive heart disease, severe valvular disease and other causes of secondary left ventricle (LV) hypertrophy. We performed 11 endomyocardial biopsy, 8 intraoperative biopsy, 1 study of explanted heart, 1 autopsy with virus investigation (real-time polymerase chain reaction) of the blood and myocardium. Mutational screening had included simultaneous sequencing of the MYBPC3, TAZ, TPM1, LDB3, MYL2, ACTC1, MYL3, MYH7, TNNI3 and TNNT2 genes based on NGS technology (Ion Torrent PGMTM) with following Sanger resequencing of potentially significant genetic variants. For patients with a phenotype of particular genetic syndrome the Sanger sequencing of target gene(s) for performed first. Clinical examination had included electrocardiography, Holter monitoring, echocardiography, coronary angiography, computer tomography/magnetic resonance imaging (by indication). The mean follow-up was 8 [3;32] month.Results. Isolated HCM was found in 28 patients, and 10 have a combination of HCM and noncompaction myocardium (NCM). Mutations in the MYH7 and MYBPC3 genes were detected in six cases. In 17 cases (30.9%) the non-sarcomeric causes of LVHS were detected. Three patients had Fabry disease, 2 ‒ had Danon disease, in 10patients we found amyloidoses, in 1 – Friedreich ataxia, and 1 patient was diagnosed with LEOPARD syndrome (all cases were confirmed by DNA diagnostics). Genotype-positive diagnosis was established in 23.6% of patients. In patients with HCM were significantly more frequent asymmetric septal hypertrophy with obstruction and muscle bridges, in other forms of primary hypertrophy – right ventricular hypertrophy, low QRS voltage, QS complexes and increasing of ejection fraction (EF) (55.7±12.5% vs 62.5±10.1% in HCM, p=0.08). The morphologic signs of myocarditis were in 46.7% of patients with HCM detected: in 3 patients with NCM and in 4 patients with isolated HCM. The viral genome in the myocardium was in 11 patients with HCM (73.3%) detected, previously human herpes virus type 6 (it was correlation with myocarditis) and parvovirus B19. Eleven patients died due to a stroke/heart failure without no significant differences between patients with HCM and phenocopy.Conclusion. The spectrum of causes of the primary left ventricular hypertrophy is very wide. The frequency of myocarditis associated with sarcomeric HCM was 46.7%. When lower EF and heart failure in patients with HCM can be result of myocarditis, in patients with storage disease they are the result of disease itself.

Based on the data available in the literature, it is obvious that inflammation plays a significant role in the initiation, support and progression of AF. However, it is important that AF by itself is a factor provoking the development of inflammation in the atrial myocardium, which leads to the further progression of the disease according to the principle of “AF produces AF.” The question of the primacy of the development and correlations of process links is still not fully understood: does inflammation leads to fibrillation or does fibrillation leads to inflammation?The authors analyzed the data of the world literature and tried to describe the main concepts of the inflammatory theory of AF and the practical aspects of its application.

Loeffler's endocarditis remains is a very rare disease, develops due to eosinophilic inflammation predominantly of the endocardium with an outcome in fibrosis and massive thrombus formation and. He is generally characterized by an unfavorable prognosis. Clinical case of a 42-year-old patient with Loeffler endocarditis is presented. The development of the disease was preceded by a polyvalent allergy, mild dry eye syndrome and pansinusitis with a single eosinophilia of blood up to 16%. The reason for the hospitalization was the appearance of biventricular heart failure. During the previous year, the level of blood eosinophils remained normal, a threefold increase in the level of eosinophilic cationic protein was observed once. A 20-fold increase in the pANCA level, a 2.5-fold increase in the level of antibodies to DNA, an antibody to the nuclei of cardiomyocytes 1:160 were detected. The diagnosis was made on the basis of electrocardiography data (low QRS voltage, atrial hypertrophy), echocardiography, multispiral computed tomography and magnetic resonance imaging of the heart (thickening and delayed contrasting of the endocardium, massive thrombosis of the left ventricular apex with obliteration of its cavity, encapsulated fluid in the pericardium with compression of the right ventricle). Systolic dysfunction, severe signs of restriction and arrhythmias were absent. Trombectomy, tricuspid valve plasty, pericardial resection, suturing of an open oval window were performed. Signs of active inflammation with single eosinophils, vasculitis, perimuscular sclerosis, endocardial sclerosis were detected in morphological and immunohistochemical studies of endo-, myo-, pericardium. Viral genome was not found. The therapy with methylprednisolone 24 mg/day, azathioprine 75 mg/day was started. Six months after the operation, the symptoms of heart failure are completely absent, the thrombosis did not recur.

Aim. To study the association between morphological changes and comorbidities and atrial fibrillation (AF) catheter ablation efficacy, and to evaluate morphological predictors of AF recurrence in patients with durable pulmonary vein isolation (PVI) after catheter intervention.Material and methods. Fifty-four patients with paroxysmal/persistent AF without severe structural heart disease were enrolled in this study who underwent primary pulmonary vein cryoballoon ablation (CBA) with simultaneous implantation of an electrocardiogram loop recorder (Medtronic Reveal XT) and interatrial/interventricular septum biopsy. The follow-up duration was 12 months with scheduled 3, 6 and 12 month visits. Patients with recurrent AF were referred for redo procedure, where the PVI durability was assessed. The follow-up duration after the second procedure was also 12 months. The patients were divided into two groups: patients without recurrent AF and patients with recurrent AF after two catheter interventions.Results. In interventricular septum biopsy samples we found the morphological criteria of myocarditis in 70% of the patients (n=34). Individual morphological changes were detected in all patients. The most common types were cardiomyocyte hypertrophy — 98,1% (n= 52), overcrossing of cardiomyocytes — 77,4% (n=41) and lymphohistiocytic infiltration of the interstitium by more than 14 cells — 75,5% (n=40). Patients with persistent AF were significantly more likely to have endothelial cell swelling (55% vs 45%, p=0,022). Interstitial tissue edema was the only morphological parameter significantly associated with AF recurrence (p=0,03).Conclusion. In patients with AF and no structural heart disease who underwent drug-resistant AF cryoballoon ablation, morphological changes in the myocardium of the atria and ventricles are detected in 100% of cases. The predominant biopsy diagnosis is myocarditis. Morphological signs of the inflammatory process activity, as interstitial tissue edema are associated with the high incidence of non-venous AF. Further preoperative evaluation is needed to identify patients with non-venous atrial fibrillation, which will increase the effectiveness of interventional approach.

In patients with severe mitral regurgitation (MR), additional echocardiographic indices could be helpful to optimize surgical timing before irreversible left heart myocardial dysfunction has occurred. We investigated the correlation of left atrial (LA) strain by speckle tracking echocardiography with prognosis after mitral surgery for severe MR, and its association with LA fibrosis. 71 patients with primary severe MR undergoing pre-operative echocardiographic assessment were initially enrolled. Exclusion criteria were: other valvular disease>moderate, history of coronary artery disease, heart failure (HF), hypertrophic cardiomyopathy, left bundle branch block, previous pacemaker implantation, heart transplantation, poor acoustic window. The primary endpoint was the occurrence of composite events (HF and mortality); the secondary endpoint was post-operative functional capacity (NYHA and Borg CR10 class). LA fibrosis was assessed by atrial biopsy specimens in a subset of patients. Of 65 eligible patients, the primary endpoint occurred in 30 patients (medium follow-up: 3.7 ± 1 years for event-group, 6.8 ± 1 years for non-event group). After Kaplan-Meier analysis, peak atrial longitudinal strain (PALS) provided good risk stratification (5-year event-free survival:90 ± 5% for PALS≥21% vs 30 ± 9% for PALS<21%, p < 0.0001); it was an independent and incremental predictor of outcome in four multivariate Cox adjusted models. There was also an association between PALS and the secondary endpoint (NYHA: r2 = 0.11, p = 0.04; Borg CR10: r2 = 0.10, p = 0.02) and an inverse correlation between PALS<21% and LA fibrosis (r2 0.80, fibrosis: 76.6 ± 20.7% vs 31.9 ± 20.8%; p < 0.0001). Global PALS emerged as a reliable predictor of outcome and functional capacity for severe primary MR, and as a marker of LA fibrosis. • In severe mitral regurgitation (MR), optimal surgical timing should be recognized. • Preoperative left atrial strain could be useful to assess prognosis after MR surgery. • LA reservoir strain was a good predictor of clinical and functional outcome in MR. • LA reservoir strain was also correlated with LA fibrosis assessed by atrial biopsy. [ABSTRACT FROM AUTHOR]

Background Eosinophilic myocarditis (EM) is rare but accounts for 12–22% of histologically proven acute myocarditis cases. Acute necrotizing EM is considered an aggressive, life-threatening disease which is usually treated by high-dose corticosteroid therapy. Case summary We report the case of a 27-year-old man with acute severe pericarditic chest pain, moderately reduced left ventricular (LV) ejection fraction, and a small pericardial effusion. Troponin I level was highly elevated in the absence of coronary artery disease, leading to the diagnosis of acute myopericarditis. In the absence of blood eosinophilia and despite a negative cardiac magnetic resonance study, LV endomyocardial biopsy revealed an acute necrotizing EM. With conventional antiphlogistic and heart failure therapy, the patient became symptom-free and inflammatory and cardiac necrosis markers as well as LV ejection fraction normalized within days. Thus, in the absence of a systemic hypereosinophilic disorder, there was no need for steroid therapy. Long-term follow-up over 12 months showed sustained normalization of cardiac structure and function. Discussion Acute necrotizing eosinophilic myopericarditis is not always a dreadful cardiac disease. There are idiopathic cases which may quickly resolve without immunosuppression. There seems to be a publication bias towards critical cases. [ABSTRACT FROM AUTHOR]

Aim. To evaluate the efficiency of immunosuppressive therapy (IST) in virus-negative (V–) and virus-positive (V+) patients with lymphocytic myocarditis (LM). Subjects and methods. 60 patients (45 males) (mean age 46.7±11.8 years) with dilated cardiomyopathy (mean left ventricular (LV) end diastolic size (EDS) 6.7±0.7 cm; ejection fraction (EF) 26.2±9.1%) were examined. The diagnosis of active/borderline LM was verified by right ventricular endomyocardial biopsy in 38 patients, by intraoperative LV biopsy in 10, in the study of explanted hearts from 3 patients and at autopsy in 9. The investigators determined the genomes of parvovirus B19, herpes viruses types 1, 2 and 6, Epstein—Barr (EBV), zoster, and cytomegalovirus in the blood and myocardium and, if antibodies were present in the blood, hepatitis B and C viruses, as well as antibodies against antigens in the endothelium, cardiomyocytes and their nuclei, smooth muscles, fibers of the conducting system. IST was used in terms of histological, immune, and viral activities. IST was performed in 22 V+ patients (Group 1) and in 24 V– patients (Group 2); this was not done in 10 V+ patients (Group 3) and V– patients (Group 4). IST comprised methylprednisolone at a mean dose of 24 mg/day (n=40), hydroxychloroquine 200 mg/day (n=20), azathioprine at a mean dose of 150 mg/day (n=21); antiviral therapy included acyclovir, ganciclovir, intravenous immunoglobulin (n=24). The follow-up period was 19 (7.3—40.3) months. Results. The viral genome was detected in the myocardium of 32 patients who made up a V+ group. The degree of histological activity did not differ in relation to the presence of viral genome in the myocardium. The degree of immune activity (anticardiolipin antibody titers) in the V+ patients was as high as that in V– ones. At baseline, the V+ patients had a significantly higher LV EDS and a lower EF than the V– patients. Overall, IST only could lead to a significant increase in EF (from 26.5±0.9 to 36.0±10.8%; p

Acute lymphocytic myocarditis in pregnancy is rare, with no established management guidelines to date. A 40-year-old woman at 34 weeks of gestation complained of shortness of breath upon exertion. An electrocardiogram revealed broad ST elevation, and echocardiography showed diffuse impairment of left ventricular contractility. The patient was immediately transferred to our hospital for suspected takotsubo cardiomyopathy. We considered myocarditis based on the patient's prior cold-like symptoms and additional examination. Myocardial biopsy revealed lymphocyte infiltration, which confirmed acute lymphocytic myocarditis. Although there were no signs of heart failure or conduction disturbance under catecholamine, her hemodynamics were weak. Emergency cesarean section was performed because of possible hemodynamic failure during the remaining course of pregnancy. Both the mother and baby were discharged without any subsequent events. If acute myocarditis is suspected during pregnancy, prompt myocardial biopsy is crucial for timely pathological diagnosis and treatment decisions. Clinicians should consider premature delivery prior to a possible failure in maternal hemodynamics. < Learning objective: Acute lymphocytic myocarditis in pregnancy is rare; the condition is difficult to diagnose, and management protocols have not been established. If acute myocarditis is suspected during pregnancy, early myocardial biopsy is vital for prompt diagnosis and treatment decisions. Premature delivery should be considered if maternal hemodynamics is expected to destabilize.> [ABSTRACT FROM AUTHOR]

A 69-year-old woman with previous pancreaticoduodenectomy was admitted for evaluation of chest discomfort on effort and leg edema for a few months. Oral flosemide before admission for 1 week failed to relieve her symptoms. Her blood pressure was 105/51 mmHg and heart rate was 76 beats/min. Chest X-ray revealed an enlarged heart and mild pulmonary congestion. Echocardiography demonstrated normal left ventricular ejection fraction and diastolic dysfunction with no left ventricular hypertrophy. Cardiac catheterization showed normal coronary arteries, high cardiac index, and elevated intracardial pressures. Myocardial biopsy from the right ventricular septum revealed nearly normal findings. Cardiac magnetic resonance imaging (CMRI) showed both ventricles enlarged and increased global extracellular volume fraction (ECV) of 37%, but normal native T1 and T2 values. As she had pancreaticoduodenectomy, beriberi was suspected. Vitamin B1 significantly increased urine output and lowered intracardiac pressures and cardiac index. After 3 months of vitamin B1, CMRI exhibited that the right ventricle had decreased in size and the global ECV value had been lowered. Our case highlights that chronic beriberi may be associated with little myocardial damage. The increased ECV suggests that the diffuse expansion of extracellular space unrelated to myocardial edema might have been reversed by vitamin B1treatment. Morphological changes in the ventricles and myocardial damage by wet beriberi can be demonstrated by CMRI. < Learning objective: Chronic wet beriberi can occur in patients with previous pancreaticoduodenectomy although they eat regularly and never drink alcohol. Morphological changes in the ventricles and myocardial damage by wet beriberi can be demonstrated by cardiac magnetic resonance imaging.> [ABSTRACT FROM AUTHOR]

Desminopathy is a cardiac and skeletal myopathy caused by disease-causing variants in the desmin (DES) gene and represents a subgroup of myofibrillar myopathies, where cytoplasmic desmin-postive immunoreactivity is the pathological hallmark. We herein report a 28-year-old Japanese man who was initially diagnosed with sporadic hypertrophic cardiomyopathy with atrioventricular block at 9 years old and developed weakness in the soft palate and extremities. The myocardial tissue dissected during implantation of the ventricular-assisted device showed a dilated phase of hypertrophic cardiomyopathy and intracellular accumulation of proteinase K-resistant desmin aggregates. Genetic testing confirmed a de novo mutation of DES, which has already been linked to desminopathy. As the molecular diagnosis of desminopathy is challenging, particularly if patients show predominantly cardiac signs and a routine skeletal muscle biopsy is unavailable, these characteristic pathological findings of endomyocardial proteinase K-resistant desmin aggregates might aid in clinical practice.

Aims Giant-cell myocarditis (GCM) is a rare and often fatal form of myocarditis. Only a few reports have focused on fulminant forms. We describe the clinical characteristics, management and outcomes of GCM patients rescued by mechanical circulatory support (MCS). Methods and results The clinical features, diagnoses, treatments and outcomes of MCS-treated patients in refractory cardiogenic shock secondary to fulminant GCM admitted to eight French intensive care units (2002–2016) were analysed. We also conducted a systematic review of this topic. Thirteen patients (median age 44 [range 21–76] years, Simplified Acute Physiology Score II 55 [40–79]) in severe cardiogenic shock (median [range] left ventricular ejection fraction 15% [15–35%] and blood lactate 4 mmol/L) were placed on MCS 4 [0–28] days after hospital admission. Severe arrhythmic disturbances were frequent (77%), with six (46%) patients experiencing an electrical storm prior to MCS. Venoarterial extracorporeal membrane oxygenation was the first MCS option for 11 (85%) patients. GCM was diagnosed in five (38%) patients before transplant or death and treated with immunosuppressants; infections were the main complication (80%). Four patients died on MCS and no patient presented long-term survival free from heart transplant (nine patients, 69%). All transplanted patients were alive 1 year later and no GCM recurrence was reported after median follow-up of 42 [12–145] months. Conclusion Outcomes of fulminant GCMs may differ from those of milder forms. In this context, heart transplant might likely be the only long-term survival option. [ABSTRACT FROM AUTHOR]

Chemotherapy-related cardiac toxicity is a rare but serious adverse event in patients with cancer. Thus far, no case of serious cardiac toxicity of pemetrexed has been reported. We describe the case of a patient with advanced lung cancer and cardiomyopathy due to pemetrexed. A 59-year-old woman visited our hospital, and we found abnormal findings on a chest radiograph. She was diagnosed as having stage IV lung adenocarcinoma. Chemotherapy with cisplatin and pemetrexed every 3 weeks was initiated. After four cycles of chemotherapy, maintenance chemotherapy with pemetrexed was administered every 3 weeks. During the seventeenth cycle of pemetrexed, she had shortness of breath in her daily life. A chest radiograph showed an enlarged cardiothoracic ratio (66%), and the transthoracic echocardiogram demonstrated expansion of the left ventricle (diastolic diameter, 67 mm), severe global hypokinesis, and reduced left ventricular ejection fraction (28%). The coronary angiogram showed no coronary constriction. There was no delayed accumulation on the contrast-enhanced cardiac magnetic resonance imaging scan. After right heart catheterization, pathological results of a myocardial biopsy from the ventricular septum indicated no cardiac muscle hypertrophy, cardiac fibrosis, inflammatory cell infiltration, or myocyte disarray. Eventually, she was diagnosed as having pemetrexed-induced cardiomyopathy. Pemetrexed was discontinued, and furosemide, enalapril, and carvedilol were started. Then her symptoms and cardiac function improved. Early detection and discontinuation of causative agents are the most important treatment strategies in similar patients. Diuretics, angiotensin-conversion enzyme inhibitors, and beta-blockers may be effective for treating heart failure. [ABSTRACT FROM AUTHOR]

Background Pulmonary hypertension has been described in horses with severe equine asthma, but its effect on the right ventricle has not been fully elucidated. Objectives To evaluate right ventricular structure and function after a 1-week period of pulmonary hypertension secondary to acute exacerbation of severe equine asthma. Study design Prospective study. Methods A clinical episode of severe equine asthma was induced experimentally in six susceptible horses. Examinations in remission and on day 7 of the clinical episode included a physical examination with clinical scoring, echocardiography, arterial blood gas measurements, venous blood sampling for cardiac biomarkers, intracardiac pressure measurements, right ventricular and right atrial myocardial biopsies, airway endoscopy and bronchoalveolar lavage. After 1 month of recovery, physical examination, echocardiography and cardiac biomarker analysis were repeated. Echocardiographic and pressure measurements were compared with those in 10 healthy control horses. Results All horses developed clinical signs of acute pulmonary obstruction. Right heart pressures increased significantly. Altered right ventricular function could be detected by tissue Doppler and speckle tracking echocardiography. Cardiac troponin concentrations did not increase significantly, but were highly elevated in one horse which exercised in the paddock prior to sampling. Focal neutrophil infiltration was present in two myocardial samples. Even in remission, asthmatic horses showed a thicker right ventricular wall, an increased left ventricular end-systolic eccentricity index at chordal level and decreased right ventricular longitudinal strain compared with controls. Main limitations The induced clinical episode was rather mild and the number of horses was limited because of the invasive nature of the study. Conclusions Pulmonary obstruction in asthmatic horses induces pulmonary hypertension with right ventricular structural and functional changes. [ABSTRACT FROM AUTHOR]

Aims: There are no studies focusing on prognostic factors in giant cell myocarditis (GCM). We aimed to identify predictors of transplant-free survival in GCM.Methods and Results: We analysed the details of 46 patients with GCM (31 women, mean age 51 ± 12 years) seen at our hospital since 1991 and followed for the occurrence of cardiac death or transplantation till May 2015. The association of transplant-free survival with patient characteristics, laboratory data on admission, and myocardial histology in the 38 patients diagnosed prior to death or transplantation was examined. Altogether 26 patients died (n = 8) or underwent transplantation (n = 18) a median of 11 months following symptom onset. The 5-year estimate of transplant-free survival was 42% [95% confidence interval (CI) 35-48%]. By Cox regression analysis, the hazard ratio for death or transplantation was 0.87 (95% CI 0.75-0.99) per +5% difference in LVEF, 1.06 (95% CI 1.03-1.10) per + 1000 ng/L difference in NT-proBNP, and 4.57 (95% CI 1.63-11.28) for cardiac troponin-T above the median of 85 ng/L at presentation. The severity of necrosis and fibrosis in myocardial biopsy, graded by the consensus of two cardiac pathologists as none, mild, moderate, or severe, predicted the outcome with a hazard ratio of 7.17 (95% CI 2.29-22.40) for the presence of either necrosis or fibrosis of at least moderate extent.Conclusions: In GCM, the probability of transplant-free survival is 42% at 5 years from symptom onset. Markers of myocyte injury and cardiac dysfunction help predict the outcome. [ABSTRACT FROM AUTHOR]

Aim. To study possibility of nosological diagnosis in patients with dilated cardiomyopathy (DCMP) with use of myocardial biopsy. Material and methods. The trial enrolled 62 patients (23 females) with DCMP syndrome (end diastolic left ventricular size > 5.5 cm, ejection fraction < 55%). Mean age of the patients was 46.0±12.8 years. The examination included diagnosis of viral infections (Herpes virus, parvovirus B19), measurement of anticardial antibodies titer, 99Tc-MIBI single photon emission computed tomography of the myocardium, multislice computed tomography, MRT of the heart, coronarography, morphological study of the myocardium (n = 20) with application of polymerase chain reaction (PCR) for H.simplex viruses of types 1, 2 and 6, herpes zoster, Epstein-Barr, cytomegalovirus, parvovirus B-19, adenoviruses. The control group (20 operated patients with valvular heart disease and coronary heart disease) was examined for viral genome in the blood and myocardium. Results. Complex examination of DCMP patients showed the following distribution by nosological entuities: myocarditis (n = 41, 66.1%) including virus-positive (n = 14), primary DCMP (n = 16, 25.9%) including with non-compact myocarditis (NCM) in 3, with debute at delivery of the child - in 3. Arrhythmogenic right ventricular dysplasia combined with viral myocarditis (n = 2), genetic myopathy (n = 1) and Takayasu disease (n = 1) combined with NCM, isolated NCM (n = 1) were diagnosed in the rest cases. Morphological investigation of the myocardium was made in 20 patients: diagnosis of myocarditis and primary DCMP were made in 70% (including in 2 patients with CHD) and 20%. Detection of viral genome was 20 and 15% in the study and control group, respectively, in the myocardium - in 57.9 (test for parvovirus B19 was not made in 26%) and 65.0% (complete diagnosis).All the virus-positive patients with DCMP were diagnosed to have signs of active/borderline myocarditis. Diagnostic criteria and poor prognosis factors were defined. Conclusion. The nosological diagnosis of DCMP was made in all the examinees basing on the complex of clinical, case history and device evidence. The diagnosis was morphologically verified in 33.9% patients. Treatment approaches are developed.

Background: In patients with cardiomyopathy of unknown origin, endomyocardial biopsy provides the possibility of improved diagnosis and tailored treatment. Specific guidance has been developed based on cardiovascular centre of excellence experience but it is unknown if the benefits also extend into the tertiary care hospital setting. Methods: Endomyocardial biopsies was performed in patients with cardiomyopathy of unknown origin. The outcomes were mirrored against the current ESC recommendations. Results: A total of 57 patients with cardiomyopathy of unknown origin underwent endomyocardial biopsy with a mean age of 54 years and 28 % being women. In 17 patients (30 %), viruses were detected in the biopsy material, in 6 patients (11 %) cardiac amyloidosis was found of which 3 had also a positive test for viruses. The overall mortality rate was 18 % in the mean follow up period of 30 months, with a rate of 24 % in those with virus detection (mean FU 24 months) and 15 % in those without virus detection (mean FU 31 months. Death rates were 83 % in patients with cardiac amyloidosis (mean FU 10 months). Conclusion: We conclude that, limited by uncertainty stemming from the small number of included patients, endomyocardial biopsy may not prove to have a clinical impact on treatment decisions and outcomes in a tertiary care hospital setting. We consider cardiac amyloidosis to be an exception, since the mortality rate with or without concomitant virus load was extremely high. [ABSTRACT FROM AUTHOR]